GBS had long been defined as a neurological disorder with various sensori-motor manifestations with little attention to its psychiatric and sleep manifestations.

3. ◘ GBS is an acute monophasic post-
infectious immune-mediated
polyradiculoneuritis manifested by
motor weakness, diminished or
absent reflexes, sensory
disturbances with or without
cranial nerve affection.
- Karkare et al, Acta Neurol Scand. 2013.

7. - Goodfellow et al. Nature Reviews Neurology, 2016.
Georges Charles Gullian, (1876 – 1961)
◘ In July 1916, during the battle of
Somme (> one million wounded and
killed soldiers), in The World War I,
two soldiers were presented by an
acute flaccid paralysis and referred to
3 neurology physicians in the French
Army:
(Gullian, Barré and Strohl)

8. - Goodfellow et al. Nature Reviews Neurology, 2016.
Jean Alexander Barré, (1880 – 1967)
André Strohl, (1887 – 1977)
◘ The neurologists revealed the presence of
the flaccid paralysis, areflexia, minor
sensory affection and increased protein
in CSF without pleocytosis (dissociation
albumino-cytologique).
◘ These criteria were the main features of
the disease took their names:
Gullian, Barré, Strohl Disease

9. ◘ In 1859, the French neurologist, Jean
Baptiste Octave Landry reported
cases with acute ascending flacid
adult onset paralysis and named it,
Landry’s ascending paralysis or the
French polio.
Jean Baptiste Landry ,
(1826 – 1865)
-Landry, O. Note sur la paralysie ascendante aiguë. Gaz. Hebd. Méd. Chir, 1859.

10. 1976: GBS epidemic after Swine flu vaccination.
1982:
1984: Trials for plasma exchange.
1997: Trials of IVIG.
2016: Zika outbreak, GBS incidence >20 folds.
1956:
Charles Miller Fisher described the triad;
areflexia, ophthalmoplegia & ataxia.
Campylobacter jejune was isolated from
GBS patients.
Phase 2 JAPANESE ECULIZUMAB
TRIAL FOR GBS (Soliris) .
21, August,
2017:

12. - Fokke, et al. Brain, 2014.
Level of Diagnostic Certainty
Diagnostic criteria
4321
+/–+++Bilateral, flaccid weakness of limbs
+/–+++
Decreased or absent deep tendon reflexes in
weak limbs
+/–+++
Monophasic course and time between onset-
nadir 12 h to 28 days
+/––++CSF cell count <50/μl
+/––+/–+CSF protein concentration > normal value
+/––+/–+
NCS findings consistent with one of the
subtypes of GBS
++++Absence of alternative diagnosis for weakness

13. ◘ Relative symmetry.
◘ Mild sensory symptoms or signs
(not present in AMAN).
◘ Cranial nerve involvement,
especially bilateral facial
weakness.
◘ Autonomic dysfunction.

14. ◘ CSF: polymorphonuclear cells >50 cells per μL.
◘ Severe pulmonary dysfunction with little or no limb weakness.
◘ Severe sensory signs with little or no limb weakness.
◘ Bladder or bowel dysfunction at onset.
◘ Persistent bladder or bowel dysfunction.
◘ Fever at onset.
◘ Sharp spinal cord sensory level.
◘ Marked, persistent asymmetry of weakness.
◘ Slow progression of weakness without respiratory involvement (consider
acute or subacute onset CIDP).

16. (1) Acute Inflammatory Demyelinating Polyradiculoneuropathy
(AIDP).
(2) Acute Motor Axonal Neuropathy (AMAN).
(3) Acute Motor Sensory Axonal Neuropathy (AMSAN).

17. (1) Miller Fisher syndrome
(2) Ataxic variant (acute ataxic neuropathy).
(3) Pharyngeo-cervico-brachial variant (inverted GBS).
(4) Bi-brachial type
(5) Paraparetic.
(6) Multiple cranial neuropathy variant.
(7) Facial diplegia with paresthesias.
(8) Ropper’s variant (bilateral 6th & 7th palsy)
(9) Acute pandysautonomia.

19. ◘ Features of demyelination :
(1) Decreased motor nerve conduction velocity.
(2) Prolonged distal motor latency.
(3) Increased F-wave latency.
(4) Increased H-wave latency.
(5) Conduction blocks.
(6) Temporal dispersion.

20. ◘ No features of demyelination:
(1) One demyelinating feature in one nerve, if distal CMAP
amplitude is less than 10% lower limit of normal (LLN).
(2) Distal CMAP amplitude less than 80% LLN in at least two
nerves.
Transient motor nerve conduction block might be present

21. ◘ GBS is the most common cause of acute
flaccid paralysis with annual incidence of
1.2 – 2.3 per 100 000.
◘ In Europe and USA, the demyelinating
subtype is predominant while in Asia and
Central and South America, the axonal
type is more common.
- van den Berg et al, Nature Reviews Neurology, 2014.

22. ◘ GBS still a life-threatening disorder with
frequent morbidities, even with the best
available treatments.
◘ 20 – 30% of GBS patients develop
respiratory failure and require ventilation.
◘ Mortality rates in Europe and North
America is 3 – 7% with higher incidence
in other countries
- Islam et al. Neurology, 2010.

23. - Willison et al. Lancet, 2016.

24. - Darweesh et al. Journal of the Peripheral Nervous System, 2014.
◘ GBS patients often experience
anxiety & depressive symptoms as
well as brief reactive psychosis
attributable to the rapid disability,
pain, ICU admission, fear of need of
assisted ventilation and haziness in
the expectation about the future
prognosis.

25. ◘ GBS patients often experience sleep
problems in the form of insomnia,
RBD and OSA possibly due to
increased upper airway resistance,
weakness of the respiratory muscles,
decreased alveolar ventilation during
sleep and marked REM-related
hypotonia.
- Peralta et al. Clin Neurophysiol, 2006.

29. ◘ Patients were submitted to early CT
angiography within 24 hours followed
by urgent digital subtraction
angiography for diagnosis of the
ruptured aneurysmal site, size, shape
and number.
◘Early interventional embolization of the
aneurysms was then done either by
simple or balloon accessed coiling.
◘ 20 Adult GBS Patients fulfilling
Levels 1 or 2 Brighton Criteria
attending the Neuropsychiatry
Department, Tanta University
Hospitals in the period from 1st April
the end of December 2016.
◘ 10 Healthy Control subjects
matching the patient's age, sex, and
body mass index (BMI) were
included.

30. ◘ Patients were submitted to early CT
angiography within 24 hours followed
by urgent digital subtraction
angiography for diagnosis of the
ruptured aneurysmal site, size, shape
and number.
◘Early interventional embolization of the
aneurysms was then done either by
simple or balloon accessed coiling.
◘ GBS patients in impending need of
assisted ventilation with forced vital
capacity less than 20 ml/kg.
◘ Patients with chronic respiratory or
cardiac problems, chronic pain,
BMI>28, advanced metabolic or
endocrinal disorders, drugs affecting
sleep intake or heavy smokers.

31. OR

32. ◘ Patients were evaluated
initially at the time of hospital
admission and before
immunotherapy.
◘ Reassessment was done one
month after finishing
immunotherapy.

38. 65%
35%

39. 0
1
2
3
4
5
6

40. 0
3
6
9
12
15
18

41. 0
4
8
12
16
20
24
28
32

42. 0
4
8
12
16
20

43. 0
3
6
9
12
15
18

45. 0
3
6
9

46. 0
3
6
9
12

47. 0
2
4
6
8

48. 0
30
60
90
120
150
180

49. 20%
N3
WASO
N2
REMN1
8% 9%
37%
26%

50. 0
20
40
60
80
100
F M
50%
70%

51. 0
15
30
45
60
75
90

52. 0
4
8
12
16
20

53. 0
3
6
9
12
15
18

54. 80
83
86
89
92

55. 0
4
8
12
16
20

56. 0
8
16
24
32
40

57. 0
60
120
180
240
300

58. 0
12
24
36
48

60. r p
0.68 0.001*
0.3 -0.15
Hamilton Anxiety Scale 0.50 0.024*
MADRS -0.03 0.87
Wake after sleep onset
Multiple Sleep Latency Test
0.44 0.046*
Sleep efficiency -0.69 0.0007*
Apnea hypopnea index
Parameter
0.55 0.0117*Snore Index

61. 12
15
18
21
24
27
30
33
4.8 5.4 6 6.6 7.2
p =0.024

62. r p
0.97 <0.0001*
-0.75 0.0001*
Neuropathic Pain Scale 0.52 0.01*
MADRS 0.44 0.05*
Wake after sleep onset
Multiple Sleep Latency Test
0.84 <0.0001*
Sleep efficiency -0.89 <0.0001*
Apnea hypopnea index
Parameter
-0.34 0.13Snore Index

64. Early Late
Marked prolongation of WASO both early after hospital admission (left) as well as after I month of
immunotherapy (right).

65. ◘ EEG monitoring was done for 1
hour 3 times daily, started within
the first 48 hours after admission
until the 20th day or the
appearance of vasospasm signs.

67. are proportional with the degree of motor disability